AB0713 Calcinosis cutis - independent risk factor for all-cause mortality in SSc patients
Authors: N. Cristina, L. Groseanu, S. Petrescu, A. Balanescu, D. Opris-Belinski, D. Predeteanu, V. Bojinca, F. Berghea, I. Saulescu, D. Mazilu, A. Borangiu, M. M. Negru, C. Cobilinschi, M. Abobului, M. Duna, S. Daia-Iliescu, C. L. Constantinescu, V. Vlad, R. Ionescu
Journal: Annals of the Rheumatic Diseases (2022)

Background Calcinosis is a common and disabling complication of systemic sclerosis (SSc) with poorly understood pathogenesis and no effective treatment. Little is known about the etiology of this condition and management is essentially based on case studies and series. Objectives The objectives of this study were to describe the prevalence and characteristics of calcifications, and to investigate the relationships between calcinosis and clinical features in a population of patients with SSc. Methods This was a single center descriptive and retrospective study of patients treated at “Saint Mary” Clinical Hospital in Bucharest since January 2000 for SSc. Demographic and clinical features, including duration of disease progression, symptoms and parameters related to a specific organ involvement according to MEDS evaluation sheets, were evaluated in all patients. For testing the association between nominal variables chi-square test was performed while independent t test was used to compare the differences between subgropus.Logistic regression analysis was used to predict the risk of all-cause mortality. Results 154 SSc patients were selected in the database, from which we identified a final group of 31 (20%) patients with calcinosis related to systemic sclerosis. The calcinosis cohort comprised 25 females and 6 males, with a mean age of 52.6 (±14.3) years, most of them with diffuse subset (16/31). Mean disease duration was 5.6 years (±3.1). Mean modified Rodnan skin score (mRSS) was 9.46 (±3.4) and mean adjusted EScSG activity index in the subgroup with calcinosis was 3.6 (±1.9). 45.16% (14/31) patients from the calcinosis subgroup developed interstitial lung disease (ILD) vs 43.9%; pulmonary hypertension was seen in 51.6% (16/31) cases vs 10.5% (13/123). As expected, calcifications were closely associated with vascular (p=0.004) and gastrointestinal (p=0.001) involvement and pulmonary hypertension (p=0.049). Moreover, associations were stronger for severe gastrointestinal involvement defined as chronic intestinal pseudo-obstruction (p=0.001). Females (p=0.024), patients with digital ulcers (p=0.004), those with disease duration longer than 10 years (p=0.001), those with pulmonary hypertension (p=0.049) and patients with gastrointestinal involvement (p=0.044) presented significantly more calcinosis. There were no significant associations between calcinosis and disease activity, myositis, interstitial lung disease, type of scleroderma or autoantibodies. Furthermore, in the logistic regression equation we identified calcinosis as a risk factor for all-cause mortality in SSc patients [OR:2.607 (CI:1.062,6,397), p=0.037]. Conclusion Calcinosis cutis is a common manifestation is patients with SSc regardless of skin subset and type of autoantibodies. It seems to occur more often in patients with long-standing disease and is more commonly associated with vascular involvement such as digital ulcers and pulmonary hypertension. Furthermore, the present study has demonstrated that calcinosis could be an important prognostic factor when it comes to predict mortality. Given the fact that the management of calcinosis in scleroderma is an unmet need in almost half of patients with long-standing disease duration, systematic clinical trials are required to find effective measures to prevent this complication. References [1]Richardson C, Plaas A, Varga J. Calcinosis in Systemic Sclerosis: Updates in Pathophysiology, Evaluation, and Treatment. Curr Rheumatol Rep. 2020 Aug 27;22(10):73. doi: 10.1007/s11926-020-00951-2. PMID: 32856128. [2]Jinnin M. ‘Narrow-sense’ and ‘broad-sense’ vascular abnormalities of systemic sclerosis. Immunol Med. 2020 Sep;43(3):107-114. doi: 10.1080/25785826.2020.1754692. Epub 2020 Apr 23. PMID: 32324110. [3]Chander S, Gordon P. Soft tissue and subcutaneous calcification in connective tissue diseases. Curr Opin Rheumatol. 2012 Mar;24(2):158-64. doi: 10.1097/BOR.0b013e32834ff5cd. PMID: 22227955 Disclosure of Interests None declared